云南省川崎病流行病学调查

目的对云南省部分地区近10年来川崎病进行流行病学调查，了解云南川崎病发病、分布、流行特征及临床预后。方法采用日本一中国川崎病流行病调查表，发至全省有儿科病床的县市级以上医院，对1997年1月1日。2006年12月31日10年间住院的川崎病患儿进行填表调查。每5年1次共进行2次。结果调查表回收率95．6％，15个地州47家医院共报告川崎病1662例，云南省儿童川崎病患病率呈增高趋势，1997年为3．51／10万，而2006年达13．6／10万，平均为6．4／10万；男女性别比为1．58：1；发病年龄2月一14岁，5岁以下患儿占74．19％；共11个少数民族患儿占6．4％；全年均可发病，无明显的高峰季节。急性期冠状动脉改变为20．64％，急性期无死亡病例，再发率为0．36％。结论云南省川崎病发病率呈增高趋势，在省内有明显的区域分布，高寒地区无病例报道。川崎病冠脉病变已是儿童后天性心脏病的主要病因之一，早期诊断治疗是避免后遗症的重要措施。     

山西省2000-2004年住院川崎病病例流行病学分析

川崎病（KD）是一种病因尚未明确的以全身血管炎性病变为主的小儿发热出疹性疾病。自1967年日本川崎富作医师首次报道以来，世界各地相继有病例报道，我国部分省市也做了KD的流行病学调查，发现该病的发病率逐年上升。为此我们对山西省部分医院的住院病例进行回顾分析，以了解山西省KD的流行病学特征。     

川崎病患儿急性期冠脉扩张与血小板参数变化关系的研究

目的探讨川崎病（KD）冠状动脉异常患儿与血小板参数变化之间的关系.方法采用Xe-2100型全自动血细胞分析仪测定54例KD急性期患儿的外周血血小板数（PLT）、平均血小板体积（MPV）、血小板体积分布宽度（PDW）,并与30例健康儿童进行比较.冠状动脉扩张经心脏彩色超声心动图检查确定.结果KD患儿急性期PLT升高,PDW下降均较对照组有统计学差异（P〈0.05）.急性期冠脉扩张的患儿MPV较无冠脉扩张的患儿高,且差异有显著性（P〈0.01）.结论KD急性期血小板参数的变化与冠脉扩张有相关性,检测血小板参数的变化对KD的诊断、治疗及预后判断有一定的参考价值.     

Immunological Abnormalities in Kawasaki Disease with Coronary Artery Lesions

A review of our previous immunological studies on Kawasaki disease (KD) was undertaken. The results showed that peripheral blood macrophages/monocytes, T-cells and B-cells become activated during acute KD in terms of numerical changes in immunocompetent cells, expression of activated antigens on the cell surfaces and cytokine production. Also, during acute KD with coronary artery lesions (CALs) the numbers of macrophages/monocytes are increased. In addition, both the increased levels of tumor necrosis factor-α and shed intercellular adhesion molecule-1 in serum are more evident in KD patients with CALs than in those without. Our results further suggest that the main characteristics of the pathogenesis of KD are increased numbers of peripheral blood macrophages/monocytes with the secretion of monokines by these activated cells, and the expression of adhesion molecules on immunocompetent cells. These immune responses develop more vigorously in KD patients with CALs.     

Atypical Kawasaki disease with peripheral gangrene and myocardial infarction: Therapeutic implications

We describe a 2-month-old girl with atypical Kawasaki disease (KD) complicated by peripheral gangrene and myocardial infarction. Peripheral ischaemia leading to gangrene is a rare but serious complication of KD in infants younger than 7 months of age. Treatment has been targeted at reducing arterial inflammation, arteriospasm and thrombosis. We report the first patient with incomplete KD and peripheral ischaemia in whom therapy with prostaglandin E1 (PGE1) as vasodilating and antithrombotic agent appeared successful, restoring hand and foot perfusion without significant long-term sequelae. However, PGE1 could have supported development of myocardial infarction by shunting blood away from ischaemic areas distal to a giant coronary artery aneurysm with beginning thrombosis.Conclusion Atypical KD with peripheral gangrene appears to react favourably to treatment with PGE1, but needs careful monitoring to detect early signs of cardiac ischaemia.     

Cardiac sequelae of Kawasaki disease in Japan over 10 years

To observe the secular trend of a proportion of Kawasaki disease patients with cardiac sequelae in Japan, we analyzed patients with Kawasaki disease reported to nationwide surveys of the disease during 10.5 years from July 1982 to December 1992. Of 69 382 patients reported to the surveys, 10 596 (15.3%) were reported to have cardiac sequelae such as dilatation or stenosis of coronary arteries, myocardial infarction or valvar lesions, 1 month or more after onset. The percentage of cardiac sequelae was particularly high in males, infants younger than 1 year and children older than 5 years of age. The overall prevalence declined steadily over the observed period. However, the percentage for children older than 5 years of age did not decrease, whether treated with intravenous gamma globulin or untreated. As a consequence of the increased number of patients treated with intravenous gamma globulin, the proportion of Kawasaki disease patients with cardiac sequelae decreased annually. However, the proportion of children older than 5 years of age did not decrease.     

Coronary aneurysms in Kawasaki disease: Follow-up observation by two-dimensional echocardiography

Summary The development and regression of the coronary aneurysms in Kawasaki disease was studied with serial two-dimensional echocardiographic (2D echo) examinations. The diameter of the aneurysms at the proximal portions of the left coronary artery was measured on the 2D echo images in ten patients with Kawasaki disease, in whom left coronary aneurysms were found at the acute stage of the illness, and followed by 2D echo for longer than eight months. It was found that coronary aneurysms usually developed during the second week of the illness, reached maximal size at 3–8 weeks, and regressed gradually thereafter. Small aneurysms disappeared in several months, and those of intermediate size regressed in one to two years. Large aneurysms may remain for many years. Mural thrombi within the aneurysms were detected with 2D echo in three patients. They decreased in echodensity and eventually disappeared echographically.     

Aortic and mitral regurgitation in an infant with Kawasaki disease

Summary A fatal case of Kawasaki disease with extensive cardiac involvement in an 11-week-old boy is described. Two-dimensional echocardiography revealed enlargement of the left ventricle, left atrium, and aortic root as well as dilatation of the left main coronary artery. Cardiac catheterization revealed both aortic and mitral regurgitation and fusiform dilatation of the proximal segments of the coronary arteries. The clinical course was characterized by multi-system failure and death on day 31 of the illness. Aortic regurgitation is a very rare complication of Kawasaki disease and has been previously reported in one Japanese infant. The pathogenesis of aortic regurgitation in this disease is not known but may be due to aortitis and/or valvulitis. Kawasaki disease should be included as a cause of aortic regurgitation in infants.